{"orpha":423776,"name":"Hereditary gastric cancer","type":"Category","group":"Group of disorders","status":"active","flags":[],"synonyms":["Hereditary cancer of stomach"],"definition":"Hereditary gastric cancer refers to the occurrence of gastric cancer in a familial context and is described as two or more cases of gastric cancer in first or second degree relatives with at least one case diagnosed before the age of 50. Familial clustering is observed in 10% of all cases of gastric cancer, and includes hereditary diffuse gastric cancer (early onset diffuse-type gastric cancer), gastric adenocarcinoma and proximal polyposis of the stomach and familial intestinal gastric cancer (familial clustering of intestinal type gastric adenocarcinoma). Hereditary gastric cancer can also occur in other hereditary cancer syndromes such as Lynch syndrome, Li-Fraumeni syndrome, familial adenomatous polyposis and juvenile polyposis syndrome.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-423776/","orphanet_url":"https://www.orpha.net/en/disease/detail/423776","names":{"fr":{"name":"Cancer gastrique héréditaire","synonyms":["Cancer de l'estomac héréditaire"],"definition":"Le cancer gastrique héréditaire désigne l'apparition de cas de cancer gastrique dans un contexte familial ; on parle de cancer gastrique héréditaire lorsque deux cas ou plus de cancer gastrique ont été rapportés chez des proches au premier ou deuxième degré, dont au moins un cas diagnostiqué chez un proche de moins de 50 ans. Les cas héréditaires concernent 10 % de l'ensemble des cas de cancer gastrique et comprennent le cancer gastrique diffus héréditaire (cancer gastrique diffus à début précoce), l'adénocarcinome gastrique et polypose proximale de l'estomac et le cancer gastrique intestinal familial (cas familiaux d'adénocarcinome gastrique de type intestinal). Le cancer gastrique héréditaire peut également survenir dans le cadre d'autres syndromes cancéreux héréditaires, tels que le syndrome de Lynch, le syndrome de Li-Fraumeni, la polypose adénomateuse familiale et le syndrome de polypose juvénile."},"de":{"name":"Hereditärer Krebs des Magens","synonyms":["Hereditäres Magenkarzinom"]},"es":{"name":"Cáncer gástrico hereditario","synonyms":["Cáncer hereditario de estómago"],"definition":"Este término hace referencia a la aparición de cáncer gástrico en un contexto familiar y se describe como dos o más casos de cáncer gástrico en familiares de primer o segundo grado con al menos un caso diagnosticado antes de los 50 años de edad. En el 10% de todos los casos de cáncer gástrico se observa agrupación familiar, e incluye el cáncer gástrico difuso hereditario (cáncer gástrico de tipo difuso de inicio temprano), el adenocarcinoma gástrico y la poliposis proximal del estómago, y el cáncer gástrico intestinal familiar (agrupación familiar del adenocarcinoma gástrico de tipo intestinal). También puede presentarse cáncer gástrico hereditario en otros síndromes de cáncer hereditario, tales como el síndrome de Lynch, el síndrome de Li-Fraumeni, la poliposis adenomatosa familiar y el síndrome de poliposis juvenil."},"it":{"name":"Tumore gastrico ereditario","synonyms":["Tumore ereditario dello stomaco"],"definition":"Il tumore gastrico ereditario fa riferimento alla ricorrenza di un tumore gastrico all'interno di una stessa famiglia. Viene definito come due o più casi di tumore gastrico in consanguinei di primo o secondo grado, con almeno un caso diagnosticato prima dei 50 anni di età. Nel 10% dei casi di tumore gastrico si osserva una ricorrenza familiare, che comprende il tumore gastrico diffuso ereditario (tumore gastrico diffuso ad esordio precoce), l'adenocarcinoma gastrico e la poliposi prossimale dello stomaco (si vedano questi termini) e il tumore gastrico intestinale familiare (concentrazione familiare dell'adenocarcinoma gastrico di tipo intestinale). Il tumore gastrico ereditario può presentarsi anche nel contesto di altre sindromi tumorali ereditarie, come la sindrome di Lynch, la sindrome di Li-Fraumeni, la poliposi adenomatosa familiare e la poliposi giovanile (si vedano questi termini)."},"nl":{"name":"Hereditaire maagkanker","synonyms":["Erfelijke kanker van maag"],"definition":"Erfelijke maagkanker verwijst naar het voorkomen van maagkanker in een familiale context en wordt beschreven als twee of meer gevallen van maagkanker in verwanten van de eerste of tweede graad, waarvan minstens één geval werd gediagnosticeerd voor de leeftijd van 50 jaar. Familiale groepering wordt waargenomen in 10% van alle gevallen van maagkanker, en omvat erfelijke diffuse maagkanker (diffuus type maagkanker met vroege aanvang), gastrisch adenocarcinoom en proximale polypose van de maag (zie deze termen) en familiale intestinale maagkanker (familiale groepering van intestinaal type gastrisch adenocarcinoom). Erfelijke maagkanker kan ook voorkomen bij andere erfelijke kankersyndromen zoals syndroom van Lynch, syndroom van Li-Fraumeni, familiale adenomateuze polypose en juveniel polyposissyndroom (zie deze termen)."},"pt":{"name":"Cancro gástrico hereditário"},"pl":{"name":"Dziedziczny rak żołądka","synonyms":["Hereditary cancer of stomach"]},"cs":{"name":"Hereditární karcinom žaludku","synonyms":["Hereditární žaludeční nádor"]},"uk":{"name":"Спадковий рак шлунка","synonyms":["Спадковий рак шлунка"],"definition":"Hereditary gastric cancer refers to the occurrence of gastric cancer in a familial context and is described as two or more cases of gastric cancer in first or second degree relatives with at least one case diagnosed before the age of 50. Familial clustering is observed in 10% of all cases of gastric cancer, and includes hereditary diffuse gastric cancer (early onset diffuse-type gastric cancer), gastric adenocarcinoma and proximal polyposis of the stomach (see these terms) and familial intestinal gastric cancer (familial clustering of intestinal type gastric adenocarcinoma). Hereditary gastric cancer can also occur in other hereditary cancer syndromes such as Lynch syndrome, Li-Fraumeni syndrome, familial adenomatous polyposis and juvenile polyposis syndrome (see these terms)."},"zh":{"name":"遗传性胃癌","synonyms":["遗传性胃癌"],"definition":"遗传性胃癌是指在家族性背景下发生胃癌，被描述为一级或二级亲属中有两例或多例胃癌，其中至少一例在50岁之前被诊断。在10％的胃癌人群中观察到家族性聚集性病例，包括遗传性弥漫性胃癌（早发性弥漫型胃癌），胃腺癌和胃近端息肉病（请参阅以下术语）和家族性肠胃癌（家族聚集性肠型胃腺癌） 。遗传性胃癌也可发生在其他遗传性癌症综合征中，例如林奇综合征，李-弗劳梅尼综合征，家族性腺瘤性息肉病和少年性息肉病综合征（请参阅这些术语）。"},"tr":{"name":"Kalıtsal mide kanseri","synonyms":["Kalıtsal mide kanseri"]}},"xrefs":[{"source":"MONDO","code":"0018502","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"18502","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680075","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":423771,"name":"Rare carcinoma of stomach","classifications":[152,202]}],"children":[{"orpha":314022,"name":"Gastric adenocarcinoma and proximal polyposis of the stomach","type":"Disease"},{"orpha":26106,"name":"Hereditary diffuse gastric cancer","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}